Search PubMed⌕ Search

Biomedical subjects

V H Patterson

Publications and source records attributed to V H Patterson.

At least 19 recordsLinked to original sources
PubMed1998

Scapulothoracic arthrodesis for patients with facioscapulohumeral muscular dystrophy.

Ten scapulothoracic arthrodesis procedures were performed in six patients with facioscapulohumeral muscular dystrophy in order to improve considerably restricted activities of daily living. Four of these procedures were bilateral. The duration of follow-up ranged from 28 to 120 months. All patients reported improved function in activities of daily living. Active shoulder abduction was improved by an average of 44 degrees, and active flexion increased by 56 degrees. There was no deterioration in improved upper limb function with time. Complications included pneumothorax, atelectasis, pleural effusion and re-exploration for a segment of retained drain.

Adult↗
PubMed1998

Discordant repeat size and phenotype in Kennedy syndrome.

Previous reports in the literature have described correlation of increasing repeat length with severity of the phenotype, in Kennedy syndrome. We describe male siblings with different repeat lengths, with lack of expression of the phenotype in the sibling with the longer repeat length. The phenotype was identical to motor neurone disease. There is variability of expression in Kennedy syndrome and repeat length even in siblings cannot be taken as a conclusive indicator of severity. CAG repeat length cannot be used to predict the natural history of Kennedy disease. The diagnosis of Kennedy syndrome should be considered in male patients presenting with atypical motor neurone disease.

Diagnosis, Differential↗
PubMed1996

A double blind, placebo controlled, crossover trial of D-ribose in McArdle's disease.

To determine whether seven days oral D-ribose would improve exercise tolerance in a group of 5 patients with McArdle's disease, we performed a double blind placebo controlled crossover trial. Subjects performed weekly treadmill exercise tests with expired gas analysis until their times were reproducible. They then received 60 g D-ribose daily or placebo for seven days. Exercise testing was repeated on completion of this period. A seven day washout period then followed. Subjects then performed a new baseline exercise test prior to starting the other solution. Again after seven days the exercise test was repeated. There was no significant difference between pre-treatment exercise tests for peak oxygen consumption or level of leg fatigue. Patients did not like taking the ribose and D-Ribose does not appear to be of benefit to patients with McArdle's disease.

Adult↗
PubMed1996

The prevalence of inherited neuromuscular disease in Northern Ireland.

A comprehensive study of inherited neuromuscular disease was carried out in Northern Ireland between 1 February 1993 and 30 June 1994. Cases were ascertained from eight different sources and investigations and diagnoses were reviewed. Five hundred and forty three individuals were identified giving an overall prevalence of inherited neuromuscular disease of 1 in 2900 of the population. The prevalence of myotonic dystrophy was higher than that in most previously reported studies and accounted for 25% of all cases. Congenital myopathies were also found more frequently than expected. The overall prevalence compares well with that estimated by Emery from the reported prevalence rates of individual diseases.

Adolescent↗
PubMed1996

Angina in McArdle's disease.

McArdle's disease (myophosphorylase deficiency) results in the inability to metabolise skeletal muscle glycogen to lactate. A patient with this condition developed angina and therefore offered a unique opportunity to explore the differential expression of the defective myophosphorylase gene in skeletal and cardiac muscle.

Aged↗
PubMed1993

What people with epilepsy want from a hospital clinic.

A questionnaire was sent to 511 patients with epilepsy who were being reviewed at the clinics of two consultant neurologists. The questionnaire asked 19 questions about seizure type and how the diagnosis was given. It also asked how much information was given about the disease and advice about living with it. There were also questions about counselling and preference for hospital or community care. Over 96% returned the questionnaire. About one third said they were not told what epilepsy was, over 90% wanted more information about the disease, and about three quarters felt they had not been given enough information about the side-effects of antiepileptic drugs. Over 60% wanted to talk to someone other than a consultant about epilepsy, the most frequent person requested being a specialist nurse. Despite this, three quarters wanted to continue to attend the hospital clinic and 89% were generally satisfied with their hospital management. This survey has highlighted a number of shortcomings in the structure of our clinics. It should be possible to correct them by providing more structured information and having a nurse specialist available.

Adaptation, Psychological↗
PubMed1993

Respiratory gas exchange and metabolic responses during exercise in McArdle's disease.

During normal progressive exercise, the gas exchange anaerobic threshold occurs when CO2 production (VCO2) and ventilation (VE) increase so as to depart from a linear relationship to O2 consumption (VO2). This is thought to represent a gas exchange response to metabolic acidosis due to lactate accumulation. Patients with McArdle's disease have previously been reported to exhibit a steepened ventilatory response relative to VCO2, despite an inability to produce lactate. However, the VCO2 response has not been studied. We therefore investigated the VCO2-VO2 and VE-VO2 relationships in seven McArdle's disease patients and seven control subjects during symptom-limited maximal treadmill exercise. Analysis of gas exchange showed that whereas all control subjects had an easily identifiable anaerobic threshold, four of the patients had none and the other three displayed an attenuated threshold. The occurrence of the threshold in one patient was associated with a small rise in lactate and in another patient with an abrupt rise in leg discomfort, suggesting a pain response. Ammonia and the purine metabolite hypoxanthine were elevated during exercise in all patients, suggesting that ammonia may be a product of adenosine monophosphate degradation. Free fatty acid levels were also elevated, and a shift toward utilization of lipid may contribute to abnormal gas exchange responses. It is concluded that lactic acidosis contributes to the gas exchange anaerobic threshold but that other factors, such as discomfort, may be involved in the excess Ve seen during heavy exercise.

Adolescent↗
PubMed1987

The neurological practice of a district general hospital.

In a one year prospective study, 19% of 925 adult medical admissions to a district general hospital (the Downe Hospital, Downpatrick, Northern Ireland) were due primarily to a neurological complaint. A further 19 cases had an active neurological disorder which contributed to their hospital admission. Most were acute admissions via the General Practitioner or through the Accident and Emergency Department, a situation different from the practice of most regional neurological centres. Only 15 patients (8% of the neurological cases) were referred to a specialist centre.

Adult↗
PubMed1985

Subsets of T lymphocytes in relation to T lymphocyte function in multiple sclerosis.

T lymphocyte control of Epstein-Barr virus (EBV) infection of autologous B lymphocytes was examined in parallel to the enumeration of subpopulations of mononuclear cells in 22 multiple sclerosis (MS) patients and in 22 healthy individuals. All were seropositive for EBV. The incidence of lack of T cell control was significantly higher in patients than in controls, confirming previous published work. In the present study, we have shown in addition a significantly reduced proportion of OKT8+ cells and a significantly increased ratio of OKT4/OKT8 cells in the group of patients with lack of control. The findings point to abnormal immunoregulation in MS.

Adrenocorticotropic Hormone↗
PubMed1985

Thymectomy for myasthenia gravis.

The results of 14 years' experience in the surgical treatment of myasthenia gravis are reported. Twenty-one patients (14 female, 7 male) underwent thymectomy for myasthenia gravis between 1971 and 1984. The mean age of the patients was 33 years (range 14 - 57 years). The median duration of symptoms prior to surgery was 18 months (range 5 months to 35 years). The mean follow-up was 5.3 years. There were no post-operative deaths: 76% obtained benefit from thymectomy. The patients' age, sex, duration of symptoms and histology of the thymus gland did not correlate with the result of treatment. This series suggests that, while thymectomy is often beneficial in the treatment of myasthenia gravis, there are no accurate predictors of the outcome following surgery.

Adolescent↗
PubMed1983

Exercising muscle does not produce hypoxanthine in adenylate deaminase deficiency.

The failure of forearm exercise to increase plasma hypoxanthine in subjects with adenylate deaminase deficiency confirms this enzyme's role in hypoxanthine production by normal forearm exercise. The conversion of adenosine monophosphate (AMP) to hypoxanthine may reflect an alternative method of adenosine triphosphate (ATP) regeneration in working muscle.

AMP Deaminase↗
PubMed1982

Rod- and cone-mediated visual function in multiple sclerosis.

Visual thresholds and perceptual latencies were determined in patients with multiple sclerosis (MS) and in normal control subjects. Measurements were made under light- and dark-adapted conditions, with stimuli chosen to stimulate rod and cone receptors selectively. More abnormalities in perceptual latency and luminance threshold were recorded in the light-adapted condition than in the dark-adapted condition, but this result was not specific to the rod or cone systems. Possible underlying pathophysiological processes are discussed, and it is suggested that reduced conduction velocity in the demyelinated visual pathway is the most likely explanation of the observed perceptual delays and that there is no evident retinal contribution.

Adult↗